I write to announce the Colorado Chiari Institute at The Medical Center of Aurora. The Institute is focused on the care of persons 14 years or older with Chiari I malformation and those with intractable symptoms following previous Chiari surgery.

In order to provide high quality care to persons with the Chiari I malformation, requirements prior to scheduling an appointment include:

An evaluation by the primary care physician within the prior 6 months.

A neurology evaluation within the prior 6 months.

The report of an MRI of the brain or cervical spine within the prior 6 months which shows a Chiari malformation.

HgA1C, CRP, and a Vitamin D level.

A referral to the Institute can come from the patient’s primary care physician, neurologist, or neurosurgeon.

The Medical Center of Aurora, the only 2016 Leapfrog General Hospital in Colorado, provides a wide array of specialists as needed. TMCA is a two-time recipient of the Magnet designation for excellence in nursing and quality patient care.

The Colorado Chiari Institute is located in the 1400 building at The Medical Center of Aurora. The Institute address is:

The goal of surgical decompression in persons with intractable symptoms from the Chiari I malformation is to alleviate crowding caused by cerebellar tonsillar herniation in the region of the foramen magnum. Expanding the posterior cranio-cervical volume can improve CSF flow and alleviate symptoms.

However, the correlation between the amount of CSF space surgically created and clinical improvement has not been well studied. In part, this due to the tedious nature of the current tools used to measure the pre- and post-operative posterior fossa and CSF space volumes.

In an article published online on in the Journal of Neurosurgery: Pediatrics on February 17, 2017, researchers from the Division of Neurosurgery, Children's National Health System in Washington DC, describe the development of a “semiautomated program for calculating the 3D posterior fossa CSF volume.” Using this technique, the authors determined the correlation of the posterior fossa and CSF space volumes (cisterna magna, prepontine cistern, and fourth ventricle) with the clinical outcome in 42 pediatric patients undergoing decompression surgery for CMI.

Posterior fossa and CSF volumes were measured on the axial T2-weighted MRI images on scans taken before and after surgical decompression. The change in these volumes were then correlated with the postoperative outcome in “headache, syrinx, tonsillar descent, cervicomedullary kinking, and overall surgical success.”

The study revealed that greater enlargement of the posterior fossa volume, resulted in greater clinical improvement. In addition, enlargement of the cisterna magna also resulted in improved outcome. Furthermore, enlargement of the lower portion of the posterior fossa correlated with reduction in syrinx size. In authors words:

“A statistically significant association was found between a larger increase in the total posterior fossa volume and the cisterna magna CSF volume after CM-I decompression and improvement in headache, tonsillar descent, and surgical outcome. When the caudal portion of the posterior fossa volume was isolated, larger volume increases were associated with statistically significant increases in syrinx and cervicomedullary kinking in addition to headache, tonsillar descent, and surgical outcome.”

The authors also identified an area of future study: How large is large enough?

“Decompression volumes will need to be correlated with clinical outcomes in a prospective study before conclusions can be made on the optimal decompression size or technique.”

This year's ASAP Chiari & Syringomyelia Conference will be held in Long Island, NY on July 19 - 23. The image below from the ASAP newsletter shows only small part of the extensive list of speakers that have agreed to participate. Learn more here.

The Chiari & Syringomyelia Foundation (CSF) is dedicated to “finding a cure for Chiari malformation (CM), Syringomyelia (SM) and related disorders.” CSF announcements for January 2016 include the expansion of the unite@night walks and the scheduling of two educational lectures.

unite@night walks to run from May through August

CSF’s “one-mile, casual evening walks around the country” are community activities “that bring together people who are living with the devastating effects of Chiari malformation, syringomyelia, and related disorders.” The walks help support CSF’s local chapters.

This year CSF is expanding the unite@night walk season to run from May through August. To get involved, visit unitenight@CSFinfo.org or contact Cathy Poznik at cpoznik@CSFinfo.org.

“Chiari and the Mind” educational lecture by Dr. Gerald Grant

Dr. Gerald Grant, an Associate Professor of Neurosurgery at Stanford University Medical Center, has special interest in the Chiari malformations, pediatric brain tumors, epilepsy, and trauma.

On Thursday, January 28, 2016 Dr. Grant will present Chiari and the Mind at Stanford University Medical Center. The event will be held in Room 1155 at Lucile Packard Children’s Hospital. A Meet & Greet will begin at 5:30 pm and the presentation starts at 6:30 pm.

Register here or contact Cathy Poznik at cpoznik@CSFinfo.org or 330-998-6195 for questions.

Wayne Connell to present “Invisible No More®: The Seven Realities of the Invisible becoming Visible”

Wayne Connell is founder and president of the Invisible Disabilities® Association and co-author the book, But You LOOK Good, How to Encourage and Understand People Living with Illness and Pain.

Connell will be presenting Invisible No More®: The Seven Realities of the Invisible becoming Visible at the DoubleTree by Hilton Denver Tech Center in Greenwood Village, Colorado on Wednesday, February 3. Meet & Greet begins at 5:30pm and the presentation starts at 6:30 pm.

Register here or contact Cathy Poznik at cpoznik@CSFinfo.org or 330-998-6195 for questions.

The American Syringomyelia & Chiari Alliance Project (ASAP) is dedicated to clinical research and education in the Chiari I malformation and syringomyelia and the support of persons affected with these neurological disorders.

ASAP just announced the 28th Chiari & Syringomyelia Conference will be held in San Francisco, California on July 21-24, 2016. The Conference will be hosted by Gerald Grant, MD, Associate Professor of Neurosurgery at Stanford School of Medicine.

Videos from the 2015 Chiari & Syringomyelia Conference are available online

The 27th Chiari & Syringomyelia Conference held in Ypsilanti, Michigan in July 2015 was hosted by Dr. Cormac Maher, Associate Professor of Neurosurgery at the University of Michigan Health System.

Over 25 specialists presented on various topics regarding the Chiari I malformation and syringomyelia. Videos of the presentations are available on the ASAP website (flashvideo) and YouTube channel (MP4).

Pseudomeningocele (PM) is one of the more common complications following posterior fossa decompression for the Chiari I malformation (CM-I). A leak of spinal fluid through the duraplasty creates a pocket of CSF in the posterior cervical muscles. If the fluid collection enlarges, it pushes the duraplasty membrane into the foramen magnum region causing crowing and recurrence of the Chiari symptoms. In a few cases, spinal fluid leaks through the incision and, if untreated, leads to infection.

While a number of Chiari centers have been able to keep the risk of pseudomenigocele very low, rates as high as 18% patients have been reported.

If the PM is small, it can be observed with follow-up MRI scans and may resolve on its own. However, large persistent PMs pose difficulties. The duraplasty can become adherent to the underlying cerebellar tonsils and block CSF flow. Once adherent, surgical revision is difficult.

Dr. Scott Parker and colleagues at the Department of Neurological Surgery at Vanderbilt University studied the effects of symptomatic PMs on the 1-year postoperative “pain, disability, and quality of life” in patients undergoing Chiari decompression.

The researchers found that “a postoperative symptomatic pseudomeningocele has lingering effects at 1 year, which significantly diminishes the overall benefit of suboccipital decompression for CM-related symptoms.”

While the authors use this finding to argue for a “less invasive approach,” I have a different view. The less invasive approaches, such as thinning of the dura by stripping its outer layer, have a higher a risk of failure than the duraplasty approach.

The goal of surgery for the Chiari malformation is adequate posterior fossa decompression with minimal surgical risks. Thus, the key is to use a duraplasty technique that markedly decreases the risk of pseudomeningocele. This is possible through the use of autologous pericranium harvested from a separate small incision in the midline occiput just above the main incision.

Using triangular silastic templates, the appropriate size of graft can be obtained. The pericranial graft is sutured in place with a monofilament running suture in a watertight fashion. Two Valsalva maneuvers to 35 cm are performed following the repair and if any leak is visualized under microscopic magnification, the area is oversewn or a secondary patch is used.

Durplasty using a patient’s own pericranial tissue has been effective in posterior fossa decompression for patients with CM-I. The leak rate can be reduced to a minimal level: 2% or less. The use of a duraplasty results in greater expansion of the subarachnoid space at the foramen magnum than possible in non-duraplasty procedures and allows the patient the best opportunity to improve.

If a leak does develop, it is followed closely and if it enlarges, treated with lumbar drainage or surgical revision. Fortunately, the likelihood is low in centers experienced with the pericranial duraplasty technique.

A study performed at the University of Missouri School of Medicine published recently in the Journal of Neurosurgery: Pediatrics revealed the prevalence of Chiari I malformation (CM-I) in children and adolescents with autism and discussed the difficulty of recognizing CM-I the co-existence of these two disorders.

The authors evaluated the brain MRI scans of 125 pediatric patients younger than 18 years of age seen at the Thompson Center for Autism and Developmental Disorders. The Chiari I malformation was found in 5 children, their ages ranging from 3 years 9 months to 5 years. The amount of cerebellar tonsillar herniation varied from 8 to 19 mm. All five patients presented with symptoms of autism spectrum disorder (ASD) and CM-I and underwent posterior fossa decompression by surgeons at the Division of Neurological Surgery consisting of “suboccipital craniectomy, a C-1 or a C-1 and C-2 laminectomy, and duraplasty with bovine pericardium or Type I collagen allograft.”

Outcome in these 5 autistic children with CM-I was uniformly positive:

Recognizing the presence of CM-I in children with autism can be difficult. As noted in the study, symptoms of ASD include “impairments in social interaction, communication difficulties, and the presence of restricted, repetitive, and stereotyped patterns of behavior.” Frequently these impairments make it difficult to recognize other associated disorders such as CM-I. Thus, recognizing the symptoms and findings of CM-I in autistic children of is of utmost importance. Common symptoms include “headaches, ocular disturbances, sleep apnea, lower cranial nerve compression, dysphagia, dysarthria, disequilibrium with vertigo, and motor or sensory findings caused by syringomyelia or scoliosis.”

In addition:

“Features such as head holding, head banging, and difficulty in flexion and extension of the neck suggest the likelihood of headaches and neck pain. Swallowing difficulties and dysarthria may reflect lower cranial nerve dysfunction, while difficulties in balance and ambulation may reflect cerebellar or spinal cord compromise.”

The authors note the “current frequency of CM-I in the pediatric and adult population is thought to be about 1%–3.6%,” a 4% prevalence roughly similar to that in the general population.

Should routine MRI screening be performed in all children with ASD? The authors do not recommend this:

“Nevertheless, it is difficult to recommend performing cranial imaging in all patients with ASD to evaluate for CM-I, as the patient may be asymptomatic and such a practice does not represent a cost-effective form of health care delivery.”

However, they emphasize alertness on the part of evaluating physician and “clear communication” among treating specialists:

“It is therefore imperative that the physician is alert to the history given by the child’s parents or caretakers, as well as the observed clinical findings, and corroborates these aspects with the sensible utilization of further testing and subsequent management.”

Image from the Editor's files shows the median aperture of the 4th ventricle, also known as the foramen of Magendie, covered by a veil in this person with Chiari & syringomyelia. Image: CyberMed, LLC

When treating a patient with Chiari related syringomyelia by posterior fossa decompression, it is important for the neurosurgeon to open the dura and the arachnoid and evaluate the outlet of the fourth ventricle which drains CSF into the cisterna magna. The outlet called the foramen of Magendie after its discoverer is also known as the median aperture. Approximately 1 of 10 persons with Chiari/syringomyelia has a veil over the median aperture blocking or restricting CSF flow. Clearing the obstruction by microsurgically opening the veil facilitates syrinx decompression and helps avoid revision surgery.

case of a 32-year-old woman who underwent surgery for CM associated with extensive cervicothoracic syringomyelia and whose intraoperative somatosensory evoked potentials (SSEPs) did not notably improve after craniotomy or following durotomy; rather, they only improved after opening of the fourth ventricle and restoration of CSF flow through the foramen of Magendie. Postoperatively, the patient recovered completely from her preoperative neurological deficits. To the authors' knowledge, this is the first report of significant SSEP recovery after opening the fourth ventricle in the decompression of a CM-I.

When evaluating patients referred for persistent syringomyelia following posterior fossa decompression, who on MRI studies appear to have an adequate decompression, a careful review of the operative note should be performed to determine if the dura and arachnoid where opened during the initial procedure. If the dura or arachnoid had not been opened, the revision surgeon should consider exploration of the posterior fossa and examination for a possible median aperture veil (retained rhombic roof) as the preferred treatment strategy instead of placement of a syrinx stent or shunt.

Dr. Theodor Langhans, Professor and Chair of Pathological Anatomy in University of Bern, Switzerland.

Hans Chiari is appropriately recognized for describing and categorizing the Chiari malformations in reports published in 1891 and 1895. While others such as Jean Cruveilhier (1798-1874) and John Cleland (1835-1925) also made observations prior to Chiari, in an article published in the Journal of Neurosurgery: Pediatrics in March 2011 Martin M. Mortazavi and colleagues propose Theodor Langhans, the German pathologist remembered for describing “Langhans cells” in tuberculosis, as the first to describe the Chiari I malformation.

Mortazavi et al. review Theodor Langhans’ distinguished background:

“TheodorLanghans was born September 28, 1839, in Usingen (Nassau), Germany, and studied under Henle in Göttingen and von Recklinghausen in Berlin. He attended medical school in Heidelberg and, in 1864, completed his medical degree thesis on the structure of tendons in Würzburg. He was also a student under such names as Virchow, Trauber, and Frerichs. He served as assistant to von Recklinghausen until 1867. In Marburg, he collaborated with Lieberkölin and Wagner on anatomical research. He was later made Professor Ordinarius in Giessen and then moved to Switzerland in 1872 where he was appointed Professor and Chair of Pathological Anatomy in Bern, succeeding Klebs.”

In 1881, Langhans described a case of syringomyelia and “pyramidal tumors” of the cerebellum in the publicationÜber Höhlenbildung im Rückenmark als Folge Blutstauung. Mortazavi et al. translated the report (“Regarding cavity creation in the spinal cord as a consequence of obstruction to blood flow”) and believe “these were the first descriptions of what would become known as the Chiari I malformation described by Langhans as ‘pyramidal tumors.’” These selected passages are key to the description and reveal Langhans awareness of the anatomy and pathophysiology of CMI:

“In the case, which first brought to my attention the necessity to look for cavity formation in the spinal cord following a change in the cerebellar cavity, I could not find a cause for the increase in pressure; but great pressure on the pons and medulla oblongata from above was indeed apparent. Upon dissection of the cerebellum, nothing was of note except for an obvious/significant development of both tonsils, which protruded down in the form of two symmetrical pyramidal tumors and pushed the medulla oblongata in a frontal direction at almost a right angle.” (emphasis added)

“The increase in pressure in the cerebellar cavity will hinder or greatly impede the outflow of blood and cerebral spinal fluid.” (emphasis added)

“Although the association of Chiari I malformation and syringomyelia would not be commonly used until the late 20th century, it was Langhans in the 19th century who proposed this cause and effect. Therefore, appropriate recognition for this association should be given to this early pioneer who, with Chiari, helped provide us with details of hindbrain herniation that are still in use today.”

It has been known for some time that some dog breeds such as the Cavalier King Charles spaniels can have the Chiari-like malformation (CLM). Their small skulls crowd the brain and result in herniation of the cerebellum and brain stem through the foramen magnum. About 50% also have syringomyelia.

“The most important and consistent sign of CM/SM is pain, which your dog may show through crying out, withdrawn behavior, reluctance to exercise, intolerance of a neck collar or touch about the head and neck, and sleeping with the head raised. Affected dogs may have scoliosis (curvature of the spine), weakness and poor coordination and they may scratch on one side without touching the skin – called “phantom” scratching.”

Chiari-like malformation has also been found in other "toy" breeds such as Chihuahuas and the Brussels griffon. In order to determine the skull anatomy that results in CLM, a team of investigators from the United Kingdom, U.S. and Canada performed MRI measurements on skulls of “Griffon Bruxellois dogs with and without Chiari-like malformation and syringomyelia and identified several significant variables.” In the study, published recently in PLOS ONE, the authors

"... found that in the Griffon Bruxellois dog, Chiari-like malformation is characterized by an apparent shortening of the entire cranial base and possibly by increased proximity of the atlas to the occiput. As a compensatory change, there appears to be an increased height of the rostral cranial cavity with lengthening of the dorsal cranial vault and considerable reorganization of the brain parenchyma including ventral deviation of the olfactory bulbs and rostral invagination of the cerebellum under the occipital lobes."

"Our latest discoveries will be significant in driving this research forward. Our next steps will be to apply our technique to other breeds with Chiari malformation such as the Cavalier King Charles spaniel and Chihuahua."

"We also want to investigate more sensitive ways of screening so that risk of disease can be detected easier, at an earlier age and with a single MRI scan."

Chiari Medicine is an online forum for healthcare professionals caring for persons with the Chiari malformations, syringomyelia and related disorders, and for investigators pushing the science forward.